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Sjögren’s syndrome associated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treated with autologous and subsequently allogeneic haematopoietic SCT (HSCT)

Bone Marrow Transplantation volume 48, pages 1139–1140 (2013)Cite this article

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Sjögren’s syndrome (SS) is an autoimmune disease that may infrequently be associated with occasionally severe nervous system complications,1 similar to the neuropsychiatric complications of systemic lupus erythematosus. This patient developed a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to i.v. high-dose immunoglobulins, considered the gold standard therapy for this condition.2 CIDP is an autoimmune disease consisting of a sensory-motor neurological syndrome thought to be due to the interaction of cellular and humoral autoaggressive responses. The result is demyelination, leading to axonal degeneration and long-term disability. In view of the results obtained by means of autologous HSCT in a great variety of severe autoimmune diseases, the procedure has also been utilized in severe refractory cases of CIDP. The first report was published in 20023 with initially good results, but the patient relapsed 5 years later.4 Other cases, including three of his own, have been reviewed by Kazmi et al.5

Our female patient, 29 years old at the time of writing, suffered from recurrent polyarthralgias and recurrent bouts of fever from infancy, developed exudative pericarditis, and was hospitalized, and thereafter followed at the Gaslini Pediatric Clinic of the University of Genoa, Italy. At the age of 14, she was found with marked eye dryness, Raynaud’s phenomenon and positive Schirmer’s tests. Hypoechoic nodules were found in both parotids. High-titered, fine-speckled antinuclear antibodies (ANAs) were present. They were shown to consist of anti-SS-A (Ro) and anti-SS-B (La) Abs by counterimmunoelectrophoresis. At 22, she was admitted to our unit, having developed bilateral pleural effusions and an anasarcatic state without a clinically demonstrable nephropathy. She also developed a severe sensimotor polyradiculoneuropathy, which required the use of a wheelchair. A series of electromyographic examinations were indicative of a polyradiculoneuropathy with axonal demyelination, consistent with the electrodiagnostic criteria for CIDP.6 Owing to refractoriness to repeated courses of high-dose immunoglobulins, she received an auto-HSCT consisting of 304 × 106 CD34+ cells/kg, following conditioning with thiotepa (5 mg/kg) on each of the 2 consecutive days, and 100 mg/kg of CY for 2 days plus G-CSF; she had a 6-month-long remission (able to walk), relapsed and developed pancytopenia with Hb 86 g/L, WBC 11 × 109/L, Plt 12 × 109/L. BM histology/cytology was consistent with the diagnosis of severe aplastic anemia. She received an allo-SCT from her HLA-identical sister after conditioning with fludarabine 30 mg/m2 for 4 days and CY 300 mg/m2 for 4 days plus TBI 200 cGY. Engraftment was normal, with complete donor chimerism (assessed by STR-PCR profile analysis at day +34); she developed grade II GVHD limited to the skin, which responded promptly to steroid therapy. However, walking difficulties persisted, and she received an additional three cycles of high-dose immunoglobulins. At present (June 2012), the patient walks and mucosal dryness has disappeared. However, erythrocyte sedimentation rate is still high, and ANAs, of the fine-speckled type, persist with titers ranging from 1:80 to 1:320. A minimal corticotherapy (prednisolone 8 mg on alternate days) is still given. The clinical course of this patient is shown in Table 1.

Table 1 Synthesis of clinical history
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References

  1. Andonopoulos A, Lagos G, Drosos A, Montsopoulos HM . The spectrum of neurological involvement in Sjogren syndrome. Br J Rheumatol 1990; 29: 21–23.

    Article  CAS  Google Scholar 

  2. Van Schalk I, Winer JB, de Hart R, Vermeulen M . Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review. Lancet Neurol 2002; 1: 491–498.

    Article  Google Scholar 

  3. Vermeulen M, van Oers MH . Successful autologous stem cell transplantation in a patient with chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2002; 72: 127–128.

    Article  CAS  Google Scholar 

  4. Vermeulen M, van Oers MH . Relapse of chronic inflammatory demyelinating polyneuropathy 5 years after autologous stem cell transplantation. J Neurol Neurosurg Psychiatry 2007; 78: 1154.

    Article  CAS  Google Scholar 

  5. Kazmi MA, Mahdi-Rogers M, Sanvito L . Chronic inflammatory demyelinating polyradiculoneuropathy: a role for haematopoietic stem cell transplantation? Autoimmunity 2008; 43: 611–615.

    Article  Google Scholar 

  6. Van der Bergh PYK, Piéret F . Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2004; 29: 565–574.

    Article  Google Scholar 

  7. Hoyer BF, Manz RA, Radbruch A, Hiepe F . Long-lived plasma cells and their contribution to autoimmunity. Ann N Y Acad Sci 2005; 1050: 124–133.

    Article  CAS  Google Scholar 

  8. Shmerling RH . Autoantibodies in systemic lupus erythematosus: there before you know it. N Engl J Med 2003; 349: 1499–1500.

    Article  CAS  Google Scholar 

  9. Ferraccioli G, Damato R, De Vita S, Fanin R, Damiani D, Baccarani M . Hematopoietic stem cell transplantation (HSCT) in a patient with Sjögren’s syndrome and lung malt lymphoma cured lymphoma not the autoimmune disease. Ann Rheum Dis 2001; 60: 174–176.

    Article  CAS  Google Scholar 

  10. Rossi E, Catania G, Truini M, Ravetti GL, Grassia L, Marmont AM . Patients with systemic lupus erythematosus (SLE) having developed malignant lymphomas. Complete remission of lymphoma following high-dose chemotherapy, but not of SLE. Clin Exp Rheumatol 2011; 29: 555–559.

    CAS  PubMed  Google Scholar 

  11. Daikeler T, Labopin M, Di Gioia M, Abinun M, Alexander T, Miniati I et al. Secondary autoimmune disease occurring after HSCT for an autoimmune disease: a retrospective study of the EBMT Autoimmune Diseases Working Party. Blood 2011; 118: 1693–1698.

    Article  CAS  Google Scholar 

  12. Amital H, Shoenfeld Y . Autoimmunity and autoimmune diseases such as systemic lupus erythematosus. In: Lahita RG, (ed). Systemic Lupus Erythematosus. Elsevier: Amsterdam-Tokyo, 2004 pp 3–27.

    Chapter  Google Scholar 

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Authors and Affiliations

  1. Division of Haematology and Bone Marrow Transplantation, IRCCS Azienda Ospedaliera Universitaria San Martino—IST National Institute for Cancer Research, Genoa, Italy

    S Bregante, F Gualandi, M T van Lint, A Bacigalupo & A M Marmont

  2. Department of Neurosciences, Ophtalmology and Genetics, University of Genoa, Genoa, Italy

    A Schenone

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  1. S Bregante
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Correspondence to A M Marmont.

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Bregante, S., Gualandi, F., van Lint, M. et al. Sjögren’s syndrome associated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treated with autologous and subsequently allogeneic haematopoietic SCT (HSCT). Bone Marrow Transplant 48, 1139–1140 (2013). https://doi.org/10.1038/bmt.2013.18

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